A Case of Hypertrophied Left Ventricle After Successful Coarctoplasty

Coarctation of aorta(CoA) has been recognized to be cured by corrective operation, so many clinicians have discontinued follow-up early. However, high incidences of cardiovascular morbidity and mortality, of which causes have been known as persistent resting hypertension and exercise-induced hypertension after corrective operation, have been reported during long-term follow-up. And left ventricular mass increase associated with persistent resting hypertension and exercise-induced hypertension has been known as an independent risk factor of cardiovascular disease. So, even for successfully operated CoA patients, increased left ventricular mass as well as resting hypertension and exercise-induced hypertension must be detected early and normalized through persistent observation to adulthood.

Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography

PURPOSE: Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. METHODS: This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations including echocardiography were done, catheterization/angiography was performed the next day and the results were compared with those of echocardiography. The study population consisted of 9 patients, diagnosed as PAVSD in our hospital, from Jan. 1995 to Dec. 1997. RESULTS: Pulmonary blood was supplied via ductus arteriosus, in 3 patients(group 1), and in the other 6 patients, via major aortopulmonary collateral arteries(MAPCA)(group 2). The characteristic findings of group 2 were cardiac murmur heard in the back area(3 cases), and abnormal pulmonary arborization pattern(2 cases). The echocardiographic diagnosis of source of pulmonary blood flow was correct in all cases except one case-who had had two previous shunt surgeries and additional MAPCAs. In group 2, the presence of pulmonary confluence was predicted in 3 out of 4 cases. The mean number of MAPCAs found by echocardiography was 2.3+/-1.2 and by angiography, 3.5+/-1.4. CONCLUSION: In evaluation of PAVSD patients, near complete characteristics of pulmonary blood supply were identified by echocardiogram. Using it, practitioners can make decisions for shunt surgery or the need for further evaluations, including cardiac catheterization which can be more easily executed with previous echocardiographic data.

Isolated Noncompaction of the Ventricular Myocardium : Clinical features and Outcomes

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.

Flexible Bronchoscopic Finding of Congenital Lobar Emphysema

Congenital lobar emphysema is a rare disease and a cause of respiratory distress in early infancy. The most common location of pulmonary involvement is the left upper lobe. We diagnosed a case of congenital lobar emphysema in a 5-month-old female infant, who had a complex heart disease using flexible bronchoscopy. In spite of palliative heart surgery, respiratory difficulty and cyanosis did not improve and weaning from artificial ventilation was not possible. Chest X-ray and CT scan showed persistent hyperinflation in the left upper lobe. So, we performed flexible bronchoscopy. The findings showed no cartilage and patent bronchus during inspiration, but complete collapse during expiration due to a check valve effect in the left upper lobar bronchus. After excision of the involved lobe, the patient improved from respiratory distress. The observed airway patency during inspiration, and dynamic airway collapse on expiration suggest that bronchomalacia contributed to lung overinflation in this case.

Two Cases of Thin Basement Membrane Nephropathy presented with Minimal Change Nephrotic Syndrome

Thin basement membrane nephropathy(TBMN) is defined histologically as follows: 1) By light rnicroscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement rnembrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport's syndrome and systemic diseases that may affect the glomerular structure have been excluded. TBMN presented frequently with recurrent or persistent microscopic hematuria. Massive proteinuria such as in nephrotic syndrome rarely occurs in TBMN. We reported two cases of TBMN presented with typical minimal change nephrotic syndrome.

Effect of Liver Transplantation in Children with Fulminant Hepatitis

PURPOSE: Liver transplantation (LT) is regarded as an important management option for fulminant hepatitis (FH), which is associated with considerable mortality under conservative management. The aim of this study was to evaluate the outcome of children with FH according to management. METHODS: We reviewed medical records of patients presented with FH from January 1994 until April 1999. The children were grouped according to the treatment. Group A was classified for supportive treatment only and group B for supportive treatment plus LT. Children were considered as candidates for LT if the level of factor V decreased to below 20% of normal or the patient's condition deteriorated despite intensive care during the initial 48 hours. Underlying disease, duration after jaundice, grade of encephalopathy, laboratory findings, treatment and outcomes were analyzed. RESULTS: The study group comprised 7 females and 8 males aged from 8 months to 15 years old (median age of 4 years). The causes of FH were Wilson disease (4 cases), Epstein-Barr virus infection (1 case), drug (1 case) and idiopathic (9 cases). There were 5 children in group A and 10 in group B, and there were no significant differences in age, sex ratio, underlying diseases, grade of hepatic encephalopathy and laboratory findings between the two groups. One out of 5 in group A and 9 out of 10 in group B survived. But all the children in group A who met the criteria for LT and received only supportive care died. One out of 10 in group B died because of grade IVa hepatic encephalopathy which advanced to brainstem herniation. CONCLUSION: This study showed that patients who were managed with supportive care only, although LT was indicated, died and that 9 out of 10 who received LT survived. Therefore, we suggest LT should be considered in the management of FH.